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Incidence and Mortality
The majority of tumors of the paranasal sinuses present with advanced disease, and cure rates are generally poor (≤50%). Squamous cell carcinoma (SCC) is the most frequent type of malignant tumor in the nose and paranasal sinuses (70%-80%). Papillomas are distinct entities that may undergo malignant degeneration. The cancers grow within the bony confines of the sinuses and are often asymptomatic until they erode and invade adjacent structures.[1,2,3]
Nodal involvement is infrequent. Although metastases from both the nasal cavity and paranasal sinuses may occur, and distant metastases are found in 20% to 40% of patients who do not respond to treatment, locoregional recurrence accounts for the majority of cancer deaths since most patients die of direct extension into vital areas of the skull or of rapidly recurring local disease.
Cancers of the maxillary sinus are the most common of the paranasal sinus cancers. Tumors of the ethmoid sinuses, nasal vestibule, and nasal cavity are less common, and tumors of the sphenoid and frontal sinuses are rare.
The major lymphatic drainage route of the maxillary antrum is through the lateral and inferior collecting trunks to the first station submandibular, parotid, and jugulodigastric nodes and through the superoposterior trunk to retropharyngeal and jugular nodes.
Clinical Evaluation and Follow-up
Pretreatment evaluation and staging, as well as the need for multidisciplinary planning of treatment, is very important. Generally, the first opportunity to treat patients with head and neck cancers is the most effective, though occasionally salvage surgery or salvage radiation therapy, as appropriate, may be successful. Since most treatment failures occur within 2 years, the follow-up of patients must be frequent and meticulous during this period. In addition, because nearly 33% of these patients develop second primary cancers in the aerodigestive tract, a lifetime of follow-up is essential.
Carcinogenesis and Risk Factors
Some data indicate that various industrial exposures may be related to cancer of the paranasal sinus and nasal cavity. The risk of a second primary head and neck tumor is considerably increased. A subgroup has shown that paranasal sinus and nasal cavity SCC are associated with human papilloma virus (HPV) infection and that HPV-positive patients may have a better prognosis than those who are HPV-negative.
The most common cell type for paranasal sinus and nasal cavity cancers is squamous cell carcinoma. Minor salivary gland tumors comprise 10% to 15% of these neoplasms. Malignant melanoma presents in <1% of neoplasms in this region. Some 5% of cases are malignant lymphomas.[1,2]
Esthesioneuroepithelioma, sometimes confused with undifferentiated carcinoma or undifferentiated lymphoma, arises from the olfactory nerves.
Chondrosarcoma, osteosarcoma, Ewing sarcoma, and most soft tissue sarcomas have been reported for this region.
Inverting papilloma is considered a low-grade benign tumor with a tendency to recur and, in a small percentage of cases, to transform into a malignant tumor.
Midline granuloma, a progressively destructive condition, involves this region as well.
Note: The American Joint Committee on Cancer (AJCC) has published the 8th edition of the AJCC Cancer Staging Manual, which includes revisions to the staging for this disease. Implementation of the 8th edition began in January 2018. The PDQ Adult Treatment Editorial Board, which maintains this summary, is reviewing the revised staging and will make appropriate changes as needed.
The staging systems are clinical estimates of the extent of disease. The assessment of the tumor is based on inspection, palpation, and direct endoscopy when necessary. The tumor must be confirmed histologically, and any other pathological data obtained on biopsy may be included. The appropriate nodal drainage areas are examined by careful palpation. Computed tomographic and/or magnetic resonance imaging studies are generally required to adequately evaluate tumor extent prior to attempted surgical resection or definitive radiation therapy. If a patient relapses, complete restaging must be done to select the appropriate additional therapy.[1,2]
Definitions of TNM
Staging of nasal cavity and paranasal sinus carcinomas is not as well established as for other head and neck tumors. For cancer of the maxillary sinus, the nasal cavity, and the ethmoid sinus, the AJCC has designated staging by TNM (tumor, node, and metastasis) classification.
Except for T1 mucosal carcinomas, the accepted method of treatment is a combination of radiation therapy and surgery. The incidence of lymph node metastases is generally low (approximately 20% of all cases). Thus, routine radical neck dissection or elective neck radiation therapy is recommended only for patients presenting with positive nodes.
For patients with operable tumors, radical surgery is generally performed first to remove the bulk of the tumor and to establish drainage of the affected sinus(es). This is followed by postoperative radiation therapy. Some institutions continue to give a full dose of radiation therapy preoperatively for all stage II and stage III tumors and to operate 4 to 6 weeks later.[1,2,3] A review of published clinical results of radical radiation therapy for head and neck cancer suggests a significant loss of local control when the administration of radiation therapy was prolonged; therefore, lengthening of standard treatment schedules should be avoided whenever possible.
Surgical exploration may be required to determine operability.
Destruction of the base of skull (i.e., anterior cranial fossa), cavernous sinus, or the pterygoid process; infiltration of the mucous membranes of the nasopharynx; or nonresectable lymph node metastases are relative contraindications to surgery. Surgical approaches include fenestration with removal of the bulk tumor, which is usually followed by radiation therapy or block resection of the upper jaw. A combined craniofacial approach, including resection of the floor of the anterior cranial fossa is used with success in selected patients. Removal of the eye is performed if the orbit is extensively invaded by cancer. Clinically positive nodes, if resectable, may be treated with radical neck dissection.
Radiation therapy must be carried to high doses for any significant probability of permanent control. The treatment volume must include all of the maxillary antrum and involved hemiparanasal sinus and contiguous areas. The orbit and its contents are excluded except under unusual circumstances. Lymph nodes of the neck, when palpable, should be treated in conjunction with treatment of advanced carcinomas of the antrum. This may be unnecessary for early tumors.
Accumulating evidence has demonstrated a high incidence (>30%-40%) of hypothyroidism in patients who have received external-beam radiation therapy to the entire thyroid gland or to the pituitary gland. Thyroid function testing of patients should be considered prior to therapy and as part of posttreatment follow-up.[6,7]
For patients with recurrent disease, chemotherapy trials should be considered. Chemotherapy for recurrent squamous cell cancer of the head and neck has been shown to be efficacious as palliation and may improve quality of life and length of survival. Various drug combinations including cisplatin, fluorouracil, and methotrexate are effective.[8,9]
Treatment of tumors of the paranasal sinuses and of the nasal cavity should be planned on an individual basis because of the complexity involved.
Stage I disease includes small lesions.
Standard treatment options:
Current Clinical Trials
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Stage II disease includes small and moderately advanced lesions.
Standard treatment options:
Stage III disease includes small and moderately advanced lesions.
Treatment options under clinical evaluation:
Stage IV disease includes advanced lesions.
Neoadjuvant chemotherapy as employed in clinical trials has been used to shrink tumors and to render them more definitively treatable with either surgery or radiation. This chemotherapy is given prior to the other modalities; therefore, the designation of neoadjuvant is used to distinguish it from standard adjuvant therapy, which is given after or during definitive therapy with radiation or after surgery. Many drug combinations have been used in neoadjuvant chemotherapy.[6,7,8]
Chemotherapy for recurrent head and neck squamous cell cancer has shown promise. Chemotherapy may be indicated where there is recurrence in either distant or local disease after primary surgery or radiation, and when there is residual disease after primary treatment.[1,2] Survival may be improved in those achieving a complete response to chemotherapy. Combined modality therapy with platinum and radiation therapy has been used in trials such as UMCC-8810.
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
This summary was renamed from Paranasal Sinus and Nasal Cavity Cancer Treatment.
Stage Information for Paranasal Sinus and Nasal Cavity Cancer
Editorial changes were made to this section.
This summary is written and maintained by the PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.
Purpose of This Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of adult paranasal sinus and nasal cavity cancer. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
Reviewers and Updates
This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).
Board members review recently published articles each month to determine whether an article should:
Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.
The lead reviewers for Paranasal Sinus and Nasal Cavity Cancer Treatment (Adult) are:
Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.
Levels of Evidence
Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.
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The preferred citation for this PDQ summary is:
PDQ® Adult Treatment Editorial Board. PDQ Paranasal Sinus and Nasal Cavity Cancer Treatment (Adult). Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/head-and-neck/hp/adult/paranasal-sinus-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389272]
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Last Revised: 2018-02-08
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